Sweet Syndrome due to Myelodysplastic Syndrome: Possible Therapeutic Role of Intravenous Immunoglobulin in Addition to Standard Treatment.

We report an 82-year-old lady who developed sudden onset nodular and erythematous lesions and neutrophilia following an episode of urinary tract infection. Skin biopsy confirmed the diagnosis of Sweet syndrome. Response to the use of prednisolone alone was not satisfactory. The skin lesions however showed a sustained response to the regular use of intravenous immunoglobulin (IVIG) and prednisolone was slowly weaned off. Our case highlights the possible therapeutic role of IVIG in managing this condition.

Diffuse large B-cell lymphoma of the central nervous system in mycophenolate mofetil-treated patients with systemic lupus erythematosus.

Patients with systemic lupus erythematosus (SLE) are susceptible to the development of lymphoproliferative disorders and postulated causes include intrinsic defects in immune surveillance and iatrogenic administration of immunosuppressants. Since the introduction of mycophenolate mofetil (MMF) to the immunosuppressive regimen for the management of post-organ transplantation, there have been reports of primary lymphoma of the central nervous system (PCNSL). MMF has been widely used to treat active SLE patients with Class IV lupus nephritis. In addition to two previously reported cases of PCNSL among SLE patients on long-term MMF, we report a third patient who has been on treatment with MMF for 8 years. The histology showed features compatible with diffuse large B-cell lymphoma with strong immunohistochemical staining for CD20 and positive signal for Epstein-Barr virus (EBV)-encoded RNA by in-situ hybridization that is similar to other case reports, suggesting EBV driven B-cell lymphoproliferative disease. The patient responded to withdrawal of MMF, intravenous methotrexate, rituximab and whole brain radiotherapy. With the increasing use of MMF in active renal as well as non-renal exacerbations of SLE, PCNSL should be included in the differential diagnosis in patients who present with gradual onset of focal neurological deficit.

Vulvar basal cell carcinoma in China: a 13-year review.

OBJECTIVE: We conducted a 12-year retrospective review of vulvar basal cell carcinoma (BCC) in a Chinese population. STUDY DESIGN: Medical records and histopathologic reports were examined from 5 major Hospitals in Hong Kong to list all patients diagnosed with vulvar BCC. Clinical data and histologic materials were reviewed. RESULTS: Sixteen vulvar BCCs were diagnosed. Most of them were pigmented. They were removed by simple excision or wide local excision. All the carcinomas were identified in the reticular dermis. The predominant histologic pattern was nodular, which may be mistaken as adenoid cystic carcinoma. CONCLUSION: The high proportion of pigmented vulvar BCCs suggested that biopsy should be performed for any pigmented lesion in a Chinese patient. The BCCs are superficial and tissue-preserving treatment approach is recommended. The tumor depth estimation is difficult and intraoperative frozen section consultation may be helpful. Formal histopathologic assessment should be used to reach an objective diagnosis.

Penile lichen sclerosus after allogeneic stem cell transplantation.

Graft-versus-host disease (GVHD) often complicates allogeneic stem cell transplantation (SCT) and affects mainly the gut, liver, lung and skin. The microscopic morphological features of late-phase sclerodermatous chronic GVHD in the skin, namely epidermal atrophy, lymphoplasmacytic infiltration, dense dermal fibrosis and adnexal atrophy, are histologically indistinguishable from those in sporadic systemic sclerosis, morphoea and the related condition of lichen sclerosus. Mucosal orifices including those of the genitourinary system may be severely affected. We present three SCT recipients with chronic GVHD and severe posthitis leading to phimosis requiring surgery. The excised prepuces showed features of lichen sclerosus including epidermal atrophy and a subepidermal zone of eosinophilic, homogeneous and hyalinized collagen above a band-like lymphoplasmacytic infiltrate. These cases add further evidence to support the notion that penile lichen sclerosus should be included within the expanding sclerodermoid spectrum of late-stage cutaneous chronic GVHD.

A patient with congenital ichthyosis hystrix (disseminated congenital naevus) and acute lymphoblastic leukaemia.

Congenital ichthyosis is an uncommon spectrum of hereditary and sporadic hyperkeratotic epidermolytic disorders that may occur in isolation or in association with other clinical syndromes. The pattern of distribution of the hyperkeratotic lesions and family history form the basis of diagnosis. Ichthyosis may be a marker for immune deficiency and cancer prone conditions. We report a 16-year old Chinese patient with a diagnosis of ichthyosis hystrix hystrix (disseminated congenital naevus) since birth who presented with T cell acute lymphoblastic leukaemia with tetraploid clones. There are four reports of acute leukaemia complicating ichthyosis, all involving paediatric acute lymphoblastic leukaemia. The possible mechanisms involved are discussed.

Pulmonary iron overload in thalassemia major presenting as small airway disease.

Lung function abnormalities that are associated with thalassemia major are variable with etiology that is yet undetermined. Some studies have suggested that pulmonary iron deposition is a probable cause for these lung defects although there has been no antemortem histopathological and radiological evidence for this. We report a case of thalassemia major with biopsy-proven pulmonary iron overload, in which thoracic high-resolution computed tomography revealed a morphological-functional correlation consistent with small airway disease.

Primary extramedullary plasmacytoma of the lacrimal gland.

Primary extramedullary plasmacytoma (EMP) of the lacrimal gland is rare. Here we describe the histopathologic and imaging findings in a 77-year-old patient with primary extramedullary plasmacytoma of the lacrimal gland. The usefulness of CD79a in the diagnosis of plasmacytoma and the good response of the tumor to combined surgery and radiotherapy are illustrated. Plamacytoma should be included in the differential diagnosis of lacrimal tumors.

Provocation of experimental aortic inflammation and dilatation by inflammatory mediators and Chlamydia pneumoniae.

BACKGROUND: The macrophage appears to have a key role in the inflammation and proteolysis associated with the growth and development of abdominal aortic aneurysms. The role of inflammatory mediators and Chlamydia pneumoniae in stimulating the influx of macrophages and dilatation of the abdominal aorta was investigated in an experimental model. METHODS: Periaortic application of calcium chloride solution (and monocyte chemoattractant protein (MCP) 1, a cocktail of cytokines or C. pneumoniae) to the abdominal aorta of New Zealand White rabbits was performed at laparotomy. Some animals were fed a cholesterol-rich diet. The diameter of the aorta was measured by ultrasonography and after perfusion fixation, 3 weeks after laparotomy. Aortic sections were stained with RAM-11 to identify macrophages for counting. The presence of C. pneumoniae DNA was confirmed using the polymerase chain reaction. RESULTS: Aortic macrophage influx in response to MCP-1, thioglycollate or C. pneumoniae was more than doubled in the cholesterol-fed animals. In response to human recombinant MCP-1 (1 microg) the mean(s.d.) macrophage count increased from 79(19) to 340(215) per unit area (P < 0.02). Even in cholesterol-fed animals, application of MCP-1 (recombinant human or rabbit form) was not associated with aortic dilatation. Application of thioglycollate 0.1 mol/l, or live or formalin-inactivated C. pneumoniae (0.5 x 108 organisms), was associated with a similar increase in macrophages to that caused by MCP-1 and a significant (approximately twofold) increase in aortic diameter after 3 weeks. CONCLUSION: Macrophage influx into rabbit abdominal aorta, without macrophage activation, is insufficient to cause experimental aortic dilatation. C. pneumoniae antigens appeared to stimulate aortic dilatation, probably by specific activation of macrophages.

Adenoid cystic carcinoma of the breast: a tumour commonly devoid of oestrogen receptors and related proteins.

AIM: Adenoid cystic carcinoma (ACC) of the breast is an uncommon well-differentiated tumour with good prognosis, and sometimes difficult to distinguish from in-situ and invasive cribriform carcinoma (ICC) which are relatively more common. Recently, we encountered a case of ACC that proved to be totally oestrogen receptor (ER) negative by immunohistochemistry. We investigated the possibility that this may be a consistent feature that can help in differentiating ACC from ICC which are usually ER positive. METHODS AND RESULTS: The immunoperoxidase technique was used to study the expression of ER and other related proteins in six cases of ACC and two cases of ICC. All ACC cases were negative for oestrogen (ER) and progesterone (PgR) receptors whereas the two ICC were strongly positive for ER and showed a variable degree of PgR positivity. In addition, ACC cases were pS2 negative and showed minimal expression of prolactin receptors (PrlR), while the two ICC showed widespread and strong staining for pS2 and PrlR. The percentages of cells staining positively for Ki67 and p27 were generally lower in ACC than in ICC. Both tumour types were c-erbB-2 negative, but p53 was weakly to moderately positive. CONCLUSIONS: The findings suggest that a negative immunoperoxidase staining for ER would confirm the diagnosis of ACC in contrast to the positive staining which is always seen in ICC. The findings also raise the issue of the presence of a specific class of ER negative breast carcinomas which are negative not because of poor differentiation, but because of their derivation from, or differentiation along, an ER negative cell lineage.

Perivesical fat necrosis after adjuvant intravesical chemotherapy.

OBJECTIVE: To document the presence of extensive transmural and perivesical fat necrosis in a series of radical cystectomies, and associate the surgical and pathological findings with the administration of intravesical chemotherapy. PATIENTS AND METHODS: The study comprised 12 patients with pT2+ transitional cell tumours who were referred to the West Middlesex University Hospital and who proceeded to primary radical cystectomy between November 1996 and April 1998. The association between the presence of widespread transmural and extravesical necrosis and the administration of a single dose of intravesical epirubicin or mitomycin C in the 24 h after the initial transurethral resection of bladder tumour (TURBT) was analysed using the two-tailed Fisher's exact test. RESULTS: The association between the presence of transmural and extravesical fat necrosis and administration of intravesical chemotherapy was highly significant (P=0.015). CONCLUSIONS: The depth and extent of the mural muscle necrosis and perivesical fac necrosis in patients receiving intravesical chemotherapy within 24 h of TURBT is remarkable and more florid than the usual muscle necrosis seen after TURBT. Clinically, the necrotic tissue makes the cystectomy significantly more difficult technically, and may even mimic extravesical spread of malignant disease. This was not borne out by the histology of the specimens, which showed no extravesical spread of the tumour. Surgeons should be aware of the possibility that such operative findings might be the result of intravesical chemotherapy.

Polypoid rhinosinusitis in patients with host defence deficiencies: cellular infiltration and disease severity.

Polypoid rhinosinusitis is a chronic inflammatory, mucosal disease. Eosinophils may play a key role in driving and maintaining this inflammation. Polyps in conditions associated with chronic infective rhinosinusitis--such as cystic fibrosis (CF) and primary ciliary dyskinesia--however have been described as neutrophilic. We compared cell counts in polyps from 55 patients with host-defence deficiencies (HDD) to polyps from 50 patients without HDD. The CT-scan appearance was also compared to the cell counts in the HDD group. No difference was detected in the percentage of patients with eosinophils from either group. Significantly more patients in the HDD group had polyp neutrophils (p < 0.001). Non-HDD-patient polyps contain more eosinophils (p < 0.000) whilst HDD-patient polyps contained more neutrophils (p = 0.005) and plasma cells (p = 0.05). Significant correlation was found between the neutrophil count and the CT score (p = 0.012) and the mast-cell count and the CT score (p = 0.02). Eosinophils are present in HDD and non-HDD polyps. Whilst the degree of cellular infiltration may vary, to classify polyps as eosinophilic or neutrophilic may be a false distinction.

The evaluation of salivary gland tumours using proliferating cell nuclear antigen.

In order to assess its discriminating and prognostic value, we studied the immunoreactivity for proliferating cell nuclear antigen (PCNA) in tissue from 52 human salivary gland tumours using the murine monoclonal antibody PC10. The PCNA percentage count, namely, the average number of positive nuclei counted per 100 randomly selected tumour cells was recorded for each tumour. Anaplastic carcinoma was used as a positive control and histologically 'normal' salivary gland and tonsil served as a negative control. A PCNA count of 30 per cent was postulated to predict malignancy within a given salivary gland tumour i.e. a PCNA count of 30 per cent or above would indicate malignant potential. This gave a sensitivity of 96.9 per cent and a specificity of 95.2 per cent and a positive predictive value of determining malignancy of 96.8 per cent. We conclude that PCNA immunoreactivity is useful in discriminating between benign and malignant salivary gland tumours and that it may have prognostic value in this diverse group of neoplasms.